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Hypoplastic left heart syndrome

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https://www.readbyqxmd.com/read/29351346/the-congenital-heart-disease-genetic-network-study-cohort-description
#1
Thanh T Hoang, Elizabeth Goldmuntz, Amy E Roberts, Wendy K Chung, Jennie K Kline, John E Deanfield, Alessandro Giardini, Adolfo Aleman, Bruce D Gelb, Meghan Mac Neal, George A Porter, Richard Kim, Martina Brueckner, Richard P Lifton, Sharon Edman, Stacy Woyciechowski, Laura E Mitchell, A J Agopian
The Pediatric Cardiac Genomics Consortium (PCGC) designed the Congenital Heart Disease Genetic Network Study to provide phenotype and genotype data for a large congenital heart defects (CHDs) cohort. This article describes the PCGC cohort, overall and by major types of CHDs (e.g., conotruncal defects) and subtypes of conotrucal heart defects (e.g., tetralogy of Fallot) and left ventricular outflow tract obstructions (e.g., hypoplastic left heart syndrome). Cases with CHDs were recruited through ten sites, 2010-2014...
2018: PloS One
https://www.readbyqxmd.com/read/29350395/early-results-of-aortic-arch-reconstruction-and-bilateral-pulmonary-arteries-banding-modification-of-the-norwood-operation-for-treatment-of-hypoplastic-left-heart-syndrome
#2
Jacek W Pająk, Michał Buczyński, Piotr Stanek, Grzegorz Zalewski, Marek Wites, Lesław Szydłowski, Lidia Tomkiewicz-Pająk
BACKGROUND: In the period from 2003 to the end of 2015, 96 Norwood I procedures were performed in Pediatric Heart Surgery Department in Katowice, Poland, in children with hypoplastic left heart syndrome (HLHS). AIM: This paper presents a retrospective analysis of early surgical results. METHODS: The patients consisted of two groups: Group I included 59 children operated on in the years 2003-2012 in whom the stage I Norwood procedure with the Sano modification was performed with the aortic arch reconstructed by use of a homogenous pulmonary artery patch or a bovine pericardial patch...
January 19, 2018: Kardiologia Polska
https://www.readbyqxmd.com/read/29350247/prolonged-pr-interval-at-birth-predicting-the-high-occurrence-of-fatal-atrioventricular-block-in-hypoplastic-left-heart-syndrome
#3
Eiji Morihana, Kenichiro Yamamura, Yuichiro Sugitani, Hideaki Kado, Yasushi Takahata, Toshihide Nakano, Yasutaka Nakashima, Naoki Fusazaki, Shouichi Ohga
Infants with hypoplastic left heart syndrome (HLHS) are at high mortality especially when they are associated with bradyarrhythmias. However, the risk factor of developing high-grade atrioventricular block (HAVB) is still unclear. Seventy-three patients with HLHS in our institutions from 2002 to 2011 were enrolled. The survival rate was assessed by the anatomical types, treatments, occurrence of HAVB, severe tricuspid regurgitation (TR), and restrictive atrial septal defect (ASD) along with electrocardiogram findings at birth...
January 19, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29338622/the-grief-and-coping-of-parents-whose-child-has-a-constant-life-threatening-disability-hypoplastic-left-heart-syndrome-with-reference-to-the-dual-process-model
#4
https://www.readbyqxmd.com/read/29317056/a-potentially-curative-fetal-intervention-for-hypoplastic-left-heart-syndrome
#5
Andrew T Hattam
Hypoplastic left heart syndrome (HLHS) encapsulates a spectrum of complex congenital cardiovascular malformations involving varying degrees of underdevelopment of the left-sided heart structures. However, despite improved survival rates since the introduction of staged surgical reconstruction, treatment options for HLHS remain palliative rather than curative. A major limiting factor in the development of definitive curative therapy for HLHS is an incomplete understanding of its pathogenesis. Currently, the aetiology HLHS is best conceptualised by the 'flow theory' of cardiogenesis, which states that normal cardiac development is reliant on the interrelationship of normal flow patterns of blood through the developing heart, and appropriate growth of the cardiac valves and myocardium...
January 2018: Medical Hypotheses
https://www.readbyqxmd.com/read/29299618/heart-transplantation-in-children-with-turner-syndrome-analysis-of-a-linked-dataset
#6
Joshua D Chew, Jonathan H Soslow, Cary Thurm, Matt Hall, Debra A Dodd, Brian Feingold, Jill Simmons, Justin Godown
Turner syndrome (TS) patients with hypoplastic left heart syndrome (HLHS) have poor single ventricle palliation outcomes; therefore, consideration of other potential management strategies is important. Little is known about heart transplantation (HTx) in this group, as standard HTx databases do not allow for identification of TS. This study describes experiences and outcomes of HTx in TS using a unique linkage between the Scientific Registry of Transplant Recipients and the Pediatric Health Information System databases...
January 3, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29290485/tricuspid-valve-adaptation-during-the-first-interstage-period-in-hypoplastic-left-heart-syndrome
#7
Timothy Colen, Shelby Kutty, Richard B Thompson, Edythe Tham, Andrew S Mackie, Ling Li, Dongngan T Truong, Michiko Maruyama, Jeffrey F Smallhorn, Nee Scze Khoo
BACKGROUND: Tricuspid regurgitation (TR) is an important risk factor for morbidity and mortality in hypoplastic left heart syndrome (HLHS), yet the evolution of tricuspid valve (TV) dysfunction in HLHS is poorly understood. This study sought to examine changes in TV function in HLHS between the first two stages of surgical palliation and to determine the mechanism of TR at the time of stage two surgery-bidirectional cavopulmonary anastomosis (BCPA). METHODS: We prospectively investigated 44 infants at two time points-prior to Norwood-Sano (T1 - median age 5...
December 29, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29288017/improved-lung-perfusion-after-left-pulmonary-artery-patch-enlargement-during-the-norwood-operation
#8
Mona Salehi Ravesh, Jens Scheewe, Tim Attmann, Abdullah Al Bulushi, Marka-Jill Jussli-Melchers, Michael Jerosch-Herold, Dominik D Gabbert, Philip Wegner, Hans-Heiner Kramer, Carsten Rickers
BACKGROUND: Optimal pulmonary perfusion is crucial for a well-functioning Fontan circulation in patients with hypoplastic left heart syndrome (HLHS). To obtain an adequate size of the left pulmonary artery (LPA), patch enlargement is a routine part of Hemi-Fontan procedure in our center. However, LPA patch enlargement at the time of the modified Norwood procedure may have surgical advantages. Therefore, the aim of this study was to evaluate if anatomical and functional effects of the new approach are superior...
December 26, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29287139/fetal-interventions-for-structural-heart-disease
#9
David N Schidlow, Lindsay Freud, Kevin Friedman, Wayne Tworetzky
Fetal cardiac intervention (FCI) offers the potential to alter in utero anatomy and physiology. For aortic stenosis with evolving hypoplastic left heart syndrome and pulmonary atresia with intact ventricular septum with evolving hypoplastic right heart syndrome, FCI may result in maintenance of a biventricular circulation, thus avoiding single-ventricle palliation and its attendant complications. In the case of hypoplastic left heart syndrome with intact atrial septum, FCI may ameliorate in utero pathophysiology and portend a more favorable postnatal prognosis...
December 2017: Echocardiography
https://www.readbyqxmd.com/read/29287137/maternal-hyperoxygenation-a-potential-therapy-for-congenital-heart-disease-in-the-fetuses-a-systematic-review-of-the-current-literature
#10
Jennifer Co-Vu, Dalia Lopez-Colon, Himesh V Vyas, Natalie Weiner, Curt DeGroff
OBJECTIVES: To assess efficacy, safety, outcomes, and intrauterine complications following maternal hyperoxygenation (MH) therapy in fetuses with congenital heart disease (CHD). METHODS: A systematic review was performed following an electronic search of databases. Articles were published before January 1, 2017, in an English-language and non-English-language journals (with English translations), and included human fetuses and expectant mothers with a fetal diagnosis of CHD who received MH...
December 2017: Echocardiography
https://www.readbyqxmd.com/read/29287136/cerebrovascular-hemodynamics-in-fetuses-with-congenital-heart-disease
#11
Tingting Man, Yihua He, Ying Zhao, Lin Sun, Xiaowei Liu, Shuping Ge
BACKGROUND AND OBJECTIVE: It is hypothesized that diminished cerebral vascular resistance or the "brain sparing effect" is associated with fetuses with complex congenital heart defects (CHD) and may affect their neurodevelopmental outcome. An alternative explanation is that it is related to the location, cardiac output, pressure, and resistance in left heart obstructive CHDs. We sought to determine the effects of various left and right heart obstructive defects on the cerebral and placental hemodynamics and to evaluate the utility of these variables for the assessment and prognosis of CHDs...
December 2017: Echocardiography
https://www.readbyqxmd.com/read/29286269/landmark-lecture-perloff-lecture-tribute-to-professor-joseph-kayle-perloff-and-lessons-learned-from-him-aortopathy-in-adults-with-chd
#12
Koichiro Niwa
Marfan syndrome, bicuspid aortic valve, and/or coarctation of the aorta are associated with medial abnormalities of the ascending aortic or para-coarctation aorta. Medial abnormalities in the ascending aorta are prevalent in other type of patients with a variety of CHDs such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, and tetralogy of Fallot, encompassing a wide age range and may predispose to dilatation, aneurysm, and rapture necessitating aortic valve and root surgery...
December 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/29281616/influence-of-type-of-congenital-heart-defects-on-epithelial-lining-fluid-composition-in-infants-undergoing-cardiac-surgery-with-cardiopulmonary-bypass
#13
Manuela Simonato, Aldo Baritussio, Virgilio P Carnielli, Luca Vedovelli, Gianclaudio Falasco, Michele Salvagno, Massimo Padalino, Paola Cogo
BACKGROUND: In children with congenital heart disease (CHD), altered pulmonary circulation compromises gas exchange. Moreover, pulmonary dysfunction is a complication of cardiac surgery with cardiopulmonary bypass (CPB). No data are available on the effect of different CHDs on lung injury. The aim of this study was to analyze epithelial lining fluid (ELF) surfactant composition in children with CHD. METHODS: Tracheal aspirates (TAs) from 72 CHD children [age 2.9 (0...
December 27, 2017: Pediatric Research
https://www.readbyqxmd.com/read/29280178/relationship-between-cavum-septi-pellucidi-measurements-and-fetal-hypoplastic-left-heart-syndrome-or-dextro-transposition-of-the-great-arteries
#14
Michael Saadeh, Yili Zhao, Hadiza Galadima, Rabih Chaoui, Elena Sinkovskaya, Alfred Abuhamad
OBJECTIVES: The aim of this study was to compare the size and position of the cavum septi pellucidi (CSP) in fetuses with hypoplastic left heart syndrome (HLHS) or dextro-transposition of the great arteries (d-TGA) with healthy fetuses. METHODS: The CSP length, CSP width, and frontal lobe length were measured in 185 healthy fetuses (404 scans), 11 fetuses with HLHS (16 scans), and 11 fetuses with d-TGA (12 scans) between January 2005 and April 2016. Each measurement was compared between healthy fetuses and those with HLHS or d-TGA, controlling for the biparietal diameter...
December 27, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/29273201/hybrid-palliation-outcomes-after-the-comprehensive-stage-2-procedure
#15
Clifford L Cua, Patrick I McConnell, James M Meza, Kevin D Hill, Shuaiqi Zhang, Diane Hersey, Tara Karamlou, Jeffrey P Jacobs, Marshall L Jacobs, Mark Galantowicz
BACKGROUND: Comprehensive Stage 2 procedure (CS2) follows initial Hybrid Stage 1 palliation for patients with single ventricle physiology. Goal of this study was to describe CS2 outcomes and describe differences between survivors of CS2 (s-CS2) versus non-survivors (nons-CS2). METHODS: All patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) who underwent CS2 as index operation from 2010-2016 were included. Pre-operative, operative, and post-operative data were analyzed, stratified by operative mortality (in-hospital, or within 30 days of surgery), with univariate comparisons using a chi-square / Fisher exact test or Wilcoxon rank sum test...
December 19, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29248924/is-there-more-to-zika-complex-cardiac-disease-in-a-case-of-congenital-zika-syndrome
#16
Asimenia Angelidou, Zoe Michael, Arda Hotz, Kevin Friedman, Sitaram Emani, Kerri LaRovere, Helen Christou
The epidemic of Zika virus (ZIKV) has resulted in a surge of newborns with microcephaly and brain abnormalities. In this report, we describe the first case, to our knowledge, of congenital Zika syndrome with concomitant critical congenital heart disease. The mother had a confirmed ZIKV infection in the first trimester of pregnancy. Fetal ultrasonography at 31 weeks of gestation revealed cerebral cortical calcifications and hypoplastic left heart syndrome. The severity of brain involvement was assessed by postnatal magnetic resonance imaging and echocardiogram, and palliative surgery was performed...
December 16, 2017: Neonatology
https://www.readbyqxmd.com/read/29248162/surgery-for-hypoplastic-left-heart-syndrome-taking-the-long-view
#17
EDITORIAL
Darryl Shore
No abstract text is available yet for this article.
December 2, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29233199/hypoplastic-left-heart-in-turner-s-syndrome-a-primary-indication-for-transplant
#18
Joseph Philip, Dipankar Gupta, Mark S Bleiweis, Biagio A Pietra, Himesh V Vyas
Survival for hypoplastic left heart syndrome patients following the Norwood procedure is 71-90%. Mortality in patients with Turner's syndrome and hypoplastic left heart syndrome after conventional palliation (Norwood operation) has been reported as high as 80%. This questions the approach of traditional staged palliation. Here, we report a patient with hypoplastic left heart syndrome and Turner's syndrome bridged to orthotopic heart transplantation following a hybrid procedure.
December 13, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/29229621/stem-cell-therapy-for-congenital-heart-disease-a-systematic-review
#19
REVIEW
Diamantis I Tsilimigras, Evangelos K Oikonomou, Demetrios Moris, Dimitrios Schizas, Konstantinos P Economopoulos, Konstantinos S Mylonas
BACKGROUND: Congenital heart disease (CHD) constitutes the most prevalent and heterogeneous group of congenital anomalies. Although surgery remains the gold standard treatment modality, stem cell therapy has been gaining ground as a complimentary or alternative treatment option in certain types of CHD. The aim of this study was to present the existing published evidence and ongoing research efforts on the implementation of stem cell-based therapeutic strategies in CHD. METHODS: A systematic review was conducted by searching Medline, ClinicalTrials...
December 12, 2017: Circulation
https://www.readbyqxmd.com/read/29223842/interstage-evaluation-of-homograft-valved-right-ventricle-to-pulmonary-artery-conduits-for-palliation-of-hypoplastic-left-heart-syndrome
#20
Nefthi Sandeep, Rajesh Punn, Sowmya Balasubramanian, Shea N Smith, Olaf Reinhartz, Yulin Zhang, Gail E Wright, Lynn F Peng, Lisa Wise-Faberowski, Frank L Hanley, Doff B McElhinney
OBJECTIVE: Palliation of hypoplastic left heart syndrome with a standard nonvalved right ventricle to pulmonary artery conduit results in an inefficient circulation in part due to diastolic regurgitation. A composite right ventricle pulmonary artery conduit with a homograft valve has a hypothetical advantage of reducing regurgitation, but may differ in the propensity for stenosis because of valve remodeling. METHODS: This retrospective cohort study included 130 patients with hypoplastic left heart syndrome who underwent a modified stage 1 procedure with a right ventricle to pulmonary artery conduit from 2002 to 2015...
November 8, 2017: Journal of Thoracic and Cardiovascular Surgery
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